Suppr超能文献

两例同胞患唇腭裂、特殊面容、肠旋转不良和致命性先天性心脏病:一种新的常染色体隐性遗传病?

Cleft lip and palate, characteristic facial appearance, malrotation of the intestine, and lethal congenital heart disease in two sibs: a new autosomal recessive condition?

作者信息

McPherson E, Clemens M

机构信息

University of Pittsburgh, Pennsylvania, USA.

出版信息

Am J Med Genet. 1996 Mar 1;62(1):58-60. doi: 10.1002/(SICI)1096-8628(19960301)62:1<58::AID-AJMG12>3.0.CO;2-U.

Abstract

A chromosomally normal brother and sister shared a lethal pattern of anomalies including bilateral cleft lip and palate, hypertelorism, flat facial profile, flat occiput, complex congenital heart defect, and malrotation of the intestine. The male was large for gestational age, while his sister was normally grown. The girl had bifid thumbs, but the boy had only minor hand anomalies. These findings are not consistent with any previously recognized syndrome and represent a new condition with probable autosomal recessive inheritance.

摘要

一对染色体正常的兄妹表现出相同的致命性异常模式,包括双侧唇腭裂、眼距过宽、面部轮廓扁平、枕部扁平、复杂的先天性心脏缺陷和肠旋转不良。该男性胎儿为大于胎龄儿,而他的妹妹生长正常。女孩有拇指裂,而男孩只有轻微的手部异常。这些发现与任何先前公认的综合征均不相符,代表了一种可能为常染色体隐性遗传的新病症。

相似文献

8
Pallister-Killian and Fryns syndromes.帕利斯特-基利安综合征和弗林斯综合征。
Am J Med Genet. 1994 May 15;51(1):90. doi: 10.1002/ajmg.1320510124.
10
Fryns syndrome: a new variable multiple congenital anomaly (MCA) syndrome.
Am J Med Genet. 1983 Mar;14(3):461-6. doi: 10.1002/ajmg.1320140309.

引用本文的文献

1
Review of genetic factors in intestinal malrotation.肠旋转不良的遗传因素综述
Pediatr Surg Int. 2010 Aug;26(8):769-81. doi: 10.1007/s00383-010-2622-5. Epub 2010 Jun 13.

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验